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Upbeat nystagmus in anti-Ma2 encephalitis

Abstract

Anti-Ma2 encephalitis is a paraneoplastic disorder characterised by brainstem and/or limbic involvement. Eye movement abnormalities can occur in this condition, often with confusion or somnolence. We describe a patient with progressive oscillopsia (with upbeat nystagmus) and unsteadiness, followed by acute pancreatitis. She did not respond to immunomodulatory treatment and subsequently died of complications related to pancreatitis and sepsis. There was no tumour identified at autopsy, but the anti-Ma2 antibodies in her serum and the discovery of a brainstem-predominant inflammatory infiltrate at autopsy strongly suggest a paraneoplastic disorder. Our case illustrates that upbeat nystagmus can be a predominant feature in anti-Ma2 encephalitis; clinicians should consider testing for anti-Ma2 antibodies in patients with upbeat nystagmus of unknown cause.

  • Eye Movements
  • Paraneoplastic Syndrome
  • Clinical Neurology
  • Neuropathology
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